HAAAAE: warning signals for hereditary angioedema diagnosis

Abstract

Hereditary angioedema (HAE) presents high morbidity and mortality, and patients live with the risk of having laryngeal angioedema. HAE remains unknown to and underdiagnosed by many health care providers. A group of experts from the Brazilian Association of Allergy and Immunology (ASBAI) updated the Brazilian Guidelines for HAE Management. An acronym was created to remember the warning signals for the diagnosis of HAE. The acronym is “HAAAAE” for Heredity, recurrent Angioedema, recurrent Abdominal pain, Absence of urticaria, Absence of response to antihistamines and association with Estrogen. In the present study we began to validate this acronym through a questionnaire applied to three groups: allergists and immunologists, fellows-in-training in allergy and immunology and general practitioners. We hypothesized that the acronym would be easy to be remembered, because it is a combination of HAE (the initials for hereditary angioedema) and the 4A of the American Academy of Asthma, Allergy and Immunology (AAAAI). The questionnaire was answered by 8 allergists and immunologists, 21 fellows-in-training in allergy and immunology and 11 general practitioners. In relation to the allergists and immunologists, 71.4% had a HAE class and 57.1% took care for at least one patient with the disease in the year before. All of them knew the warning signals for HAE, but only one (12.5%) used the “HAAAAE” acronym to remember the signals. They remembered on average 66.7% of the warning signs. In relation to the fellows-in-training, 95.2% had an HAE class and 76.1% took care for at least one patient with the disease in the year before. All of them knew the warning signals for HAE, and 76.1% used the “HAAAAE” acronym to remember the signals. They remembered on average 91.3% of the warning signs. In relation to the general practitioners, 27.3% had an HAE class and 27.3% took care for at least one patient with the disease in the year before. The warning signals for HAE were known by 54.5%, and 27.3% used the “HAAAAE” acronym to remember the signals. They remembered on average 37.9% of the warning signs. The acronym “HAAAAE” was useful in remembering the warning signals for the diagnosis of HAE. The fellows-in-training in allergy and immunology showed the best performance. Teaching the “HAAAAE” acronym for allergists and immunologists who have graduated before its creation, as well as for general practitioners, can help them to remember the warning signs for HAE diagnosis.